Sciascia Savino, Baldovino Simone, Schreiber Karen, Solfietti Laura, Radin Massimo, Cuadrado Maria J, Menegatti Elisa, Erkan Doruk, Roccatello Dario
Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases- Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Hospital, University of Turin, piazza del donatore di Sangue 3, 10054 Turin, Italy.
Guy's and St Thomas' NHS Foundation Trust, London, UK.
Clin Mol Allergy. 2016 Jul 15;14:6. doi: 10.1186/s12948-016-0043-2. eCollection 2016.
Antiphospholipid syndrome (APS) is an autoimmune condition characterized by the presence of antiphospholipid antibodies (aPL) in subjects presenting with thrombosis and/or pregnancy loss. The currently used classification criteria were updated in the international consensus held in Sidney in 2005. Vascular events seem to result of local procoagulative alterations upon triggers influence (the so called "second-hit theory"), while placental thrombosis and complement activation seem to lead to pregnancy morbidity. The laboratory tests suggested by the current classification criteria include lupus anticoagulant, a functional coagulation assay, and anticardiolipin and anti-β2-glycoprotein-I antibodies, generally detected by solid phase enzyme-linked immunosorbent assay. The real challenge for treating physicians is understanding what is the actual weight of aPL in provoking clinical manifestations in each case. As thrombosis has a multi-factorial cause, each patient needs a risk-stratified approach. In this review we discuss the role of thrombotic risk assessment in primary and secondary prevention of venous and arterial thromboembolic disease in patients with APS, focusing on new antibody specificities, available risk scoring models and new coagulation assays.
抗磷脂综合征(APS)是一种自身免疫性疾病,其特征是在出现血栓形成和/或流产的患者体内存在抗磷脂抗体(aPL)。当前使用的分类标准在2005年于悉尼举行的国际共识会议上进行了更新。血管事件似乎是在触发因素影响下局部促凝改变的结果(即所谓的“二次打击理论”),而胎盘血栓形成和补体激活似乎会导致妊娠并发症。当前分类标准所建议的实验室检测包括狼疮抗凝物(一种功能性凝血检测)以及抗心磷脂抗体和抗β2糖蛋白-I抗体,通常通过固相酶联免疫吸附测定法进行检测。对于治疗医生而言,真正的挑战在于了解在每种情况下aPL在引发临床表现中实际所起的作用。由于血栓形成具有多因素病因,每位患者都需要采用风险分层方法。在本综述中,我们讨论血栓形成风险评估在APS患者静脉和动脉血栓栓塞性疾病一级和二级预防中的作用,重点关注新的抗体特异性、可用的风险评分模型以及新的凝血检测方法。